anti-Chloride Channel 1, Skeletal Muscle (CLCN1) Anticorps

The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. De plus, nous expédions Chloride Channel 1, Skeletal Muscle Kits (12) et Chloride Channel 1, Skeletal Muscle Protéines (7) et beaucoup plus de produits pour cette protéine.

afficher tous les anticorps Gène GeneID UniProt
CLCN1 12723 Q64347
CLCN1 25688 P35524
CLCN1 1180 P35523
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Top anti-Chloride Channel 1, Skeletal Muscle Anticorps sur anticorps-enligne.fr

Showing 10 out of 43 products:

Catalogue No. Reactivité Hôte Conjugué Application Images Quantité Fournisseur Livraison Prix Détails
Boeuf (Vache) Lapin Inconjugué WB WB Suggested Anti-Clcn1 Antibody Titration:  0.2-1 ug/ml  ELISA Titer:  1:62500  Positive Control:  Rat Brain Lanes:   1: 20ug mouse muscle membrane fraction, 2: Empty, 3: 20ug mouse brain membrane fraction, 4: Empty, 5: 20ug mouse muscle cytosolic fraction, 6: 20ug mouse brain cytosolic fraction  Primary Antibody Dilution:   1:1000  Secondary Antibody:   Anti-rabbit HRP  Secondary Antibody Dilution:   1:2000  Gene Name:   Clcn1  Submitted by:   Anonymous 100 μL Connectez-vous pour afficher 2 to 3 Days
$289.00
Détails
Humain Lapin Inconjugué WB Western blot analysis of CLCN1 expression in Hela (A), NIH3T3 (B), H9C2 (C) whole cell lysates. 200 μL Connectez-vous pour afficher 13 to 14 Days
$487.50
Détails
Chien Lapin Inconjugué WB 50 μg Connectez-vous pour afficher 7 to 9 Days
$551.83
Détails
Humain Lapin Inconjugué ELISA, WB Western blot analysis of CLCN1 using K562 whole cell lysates 100 μL Connectez-vous pour afficher 11 to 12 Days
$390.77
Détails
Humain Lapin Inconjugué WB Western blot analysis of extracts of various cell lines, using CLCN1 antibody (ABIN5973365) at 1/1000 dilution. 100 μL Connectez-vous pour afficher 11 to 16 Days
$426.40
Détails
Souris Lapin Inconjugué IHC, WB Western Blot: CLCN1 Antibody [NBP1-69124] - Rat Brain lysate, concentration 0.2-1 ug/ml. Western Blot: CLCN1 Antibody [NBP1-69124] - Lanes: 1: 20 ug mouse muscle membrane fraction, 2: Empty, 3: 20 ug mouse brain membrane fraction, 4: Empty, 5: 20 ug mouse muscle cytosolic fraction, 6: 20 ug mouse brain cytosolic fraction Primary Antibody Dilution: 1:1000 Secondary Antibody: Anti-rabbit 100 μL Connectez-vous pour afficher 8 to 11 Days
$485.88
Détails
Humain Lapin Inconjugué IHC, WB Western blot analysis of extracts of 293T and mouse liver cell lines, using CLCN1 antibody. 100 μL Connectez-vous pour afficher 16 Days
$334.77
Détails
Humain Lapin Inconjugué WB Clcn1 antibody used at 5 ug/ml to detect target protein. 50 μg Connectez-vous pour afficher 6 to 8 Days
$473.93
Détails
Humain Lapin Inconjugué WB Western blot analysis CLCN1 using K562 whole cell lysates. 100 μg Connectez-vous pour afficher 11 to 18 Days
$454.83
Détails
Humain Lapin Inconjugué IHC, WB   100 μL Connectez-vous pour afficher 7 to 8 Days
$454.67
Détails

Plus d’anticorps contre Chloride Channel 1, Skeletal Muscle partenaires d’interaction

Mouse (Murine) Chloride Channel 1, Skeletal Muscle (CLCN1) interaction partners

  1. Data show that Muscleblind-like 1 (Mbnl1 (Montrer MBNL1 Anticorps)) and Muscleblind-Like 3 (Mbnl3 (Montrer MBNL3 Anticorps)) bind skeletal muscle chloride channel (Montrer CLCA1 Anticorps) CIC-1 (Clc-1) mRNA.

  2. Sex hormones at high concentration can rapidly modulate ClC-1 in mouse skeletal muscle fibers in vitro.

  3. Myotonia (delayed muscle relaxation) is the most commonly observed symptom in DM1 patients and is caused by aberrant splicing of the skeletal muscle chloride channel (Montrer CLCA1 Anticorps) (CLCN1) gene

  4. Myotonia in adult human skeletal actin transgenic mice may be explained on the basis of a mosaic expression of ClC-1 channels in different fibres and/or on alterations of other conductances.

  5. The expression of the muscle chloride channel (Montrer CLCA1 Anticorps), ClC-1, in Huntington disease (Montrer HTT Anticorps) muscle was compromised by improper splicing and a corresponding reduction in total Clcn1 (gene for ClC-1) mRNA.

  6. the majority of functional ClC-1 channels localize to the sarcolemma and provide essential insight into the basis of myofiber excitability in normal and diseased skeletal muscle.

  7. Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel (Montrer CLCA1 Anticorps) pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy

  8. The A331T mutation causes an unprecedented alteration of ClC-1 gating and reveals novel processes defining transitions between open and closed states in ClC (Montrer CLC Anticorps) chloride channels

  9. A distinct autosomal recessive myotonic mouse in the C57BL/6 background (line B6MT)is reported in which the Clc-1 gene shows polymorphism with no functional consequences.

  10. CLC-1 deficiency not only affects muscle relaxation (myotonia) but also modulates diaphragm performance during the contractile phase of the contraction-relaxation cycle

Human Chloride Channel 1, Skeletal Muscle (CLCN1) interaction partners

  1. Loss-of- function mutations in the CLCN1 gene located on 7q35, are the primary contributors to the pathogenesis of Thomsen myotonia

  2. Regulation of CLC-1 chloride channel (Montrer CLCA1 Anticorps) biosynthesis by FKBP8 (Montrer FKBP8 Anticorps) and Hsp90beta (Montrer HSP90AB1 Anticorps) as a molecular model for myotonia congenita has been described.

  3. the spectrum of CLCN1 mutations in patients with Myotonia Congenita

  4. Combining our results with the literature on Chinese populations indicates that 21 mutations in CLCN1 have been associated with myotonia congenital, while 7 mutations in SCN4A (Montrer SCN4A Anticorps) have been associated with paramyotonia congenita, 2 mutations in SCN4A (Montrer SCN4A Anticorps) have been associated with sodium channel myotonias.

  5. report a novel ClC-1 mutation, T335N, that is associated with a mild phenotype

  6. The present study is the first demonstration of ClC-1 regulation in active human muscle, and it provides a detailed description of the involvement of PKC (Montrer PRRT2 Anticorps) and ClC-1 in the down-regulation of Gm during AP-firing activity in human skeletal muscle fibres

  7. This study, novel mutations in CLCN1 were detected, and the spectrum of CLCN1 mutations known to be associated with MC was expanded.

  8. our study confirms the presence of the myotonia causing CLCN1 mutations p.F167L and p.R105C in the Costa Rican population.

  9. we characterized three other myotonic ClC-1 mutations.

  10. In 4 patients (3 families) with recessive MC, 4 CLCN1 variants were found, 3 of which are new. c.244A>G (p.T82A) and c.1357C>T (p.R453W) were compound heterozygotes with c.568GG>TC (p.G190S). The new c.809G>T (p.G270V) was homozygous.

Chloride Channel 1, Skeletal Muscle (CLCN1) profil antigène

Profil protéine

The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen). Alternative splicing results in multiple transcript variants.

Gene names and symbols associated with CLCN1

  • chloride voltage-gated channel 1 (CLCN1) anticorps
  • chloride channel, voltage-sensitive 1a (clcn1a) anticorps
  • chloride channel protein 1 (LOC100550479) anticorps
  • chloride channel protein 1 (LOC703944) anticorps
  • chloride channel, voltage-sensitive 1 (Clcn1) anticorps
  • chloride voltage-gated channel 1 (Clcn1) anticorps
  • adr anticorps
  • Clc-1 anticorps
  • CLC1 anticorps
  • CLCN1 anticorps
  • mto anticorps
  • myotonia anticorps
  • nmf355 anticorps
  • si:dkey-14o18.5 anticorps
  • SMCC anticorps
  • SMCC1 anticorps

Protein level used designations for CLCN1

chloride channel 1, skeletal muscle , chloride channel 1, skeletal muscle (Thomsen disease, autosomal dominant) , chloride channel protein 1-like , similar to chloride channel 1, skeletal muscle (Thomsen disease, autosomal dominant) , arrested development of righting response , chloride channel protein 1 , chloride channel protein, skeletal muscle , chloride channel 1, skeletal muscle C-type , clC-1 , skeletal muscle chloride channel 1 , skeletal muscle chloride channel ClC-1

GENE ID SPECIES
100011971 Monodelphis domestica
100050692 Equus caballus
100349073 Oryctolagus cuniculus
100402136 Callithrix jacchus
472560 Pan troglodytes
795133 Danio rerio
100550479 Meleagris gallopavo
100601183 Nomascus leucogenys
703944 Macaca mulatta
12723 Mus musculus
514597 Bos taurus
25688 Rattus norvegicus
1180 Homo sapiens
403723 Canis lupus familiaris
427949 Gallus gallus
100733719 Cavia porcellus
100520812 Sus scrofa
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